title: Autoimmune hepatitis in children: experiences in a tertiary center.
abstract: OBJECTIVE
  Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology that occurs in the children of all ages. The present study aimed to evaluate the clinical and paraclinical presentations, including pattern of autoantibodies, response to treatment, mortality, and liver transplantation outcome in the Iranian children with AIH.
  
  
  METHODS
  The medical records of 87 children (56 girls and 31 boy) diagnosed with AIH between 2001 and 2010 were retrospectively analyzed for clinical and paraclinical profiles and also treatment outcome.
  
  
  RESULTS
  The mean age of the patients was 10.1±4.5 years (64.4% females). The most common clinical findings were jaundice (70.1%), splenomegaly (67.8%), and hepatomegaly (51.7%). Antinuclear, anti-smooth muscle, and anti LKM antibodies were positive in 14/62, 22/53 and 6/40 patients, respectively (36 patients had type 1 AIH, 6 patients had type 2 AIH, 26 patients were seronegative, and autoantibodies were not available in 19 cases). The most common histological finding in the liver biopsies was chronic hepatitis with interface activity that was seen in 65 (74.7%) patients. The complete response was seen in 52 (59.8%) patients and 24 (27.6%) patients underwent liver transplantation. One-year and five-year survival rates were 87.5% and 80% in the transplanted patients.
  
  
  CONCLUSIONS
  AIH should be kept in mind in the differential diagnosis of both acute and chronic liver diseases in the children and treatment with combination of corticosteroids and azathioprine is a good treatment option. In the patients with end stage liver cirrhosis that did not respond to medical therapy, liver transplantation is the treatment of choice.
authors.affiliation: Gastroenterohepatology Research Center, Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran ; Shiraz Transplant Research Center, Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Seyed-Mohsen
authors.initials: SM
authors.lastname: Dehghani
authors.forename: Mahmood
authors.initials: M
authors.lastname: Haghighat
authors.forename: Mohammad-Hadi
authors.initials: MH
authors.lastname: Imanieh
authors.forename: Nasser
authors.initials: N
authors.lastname: Honar
authors.forename: Amir-Masoud
authors.initials: AM
authors.lastname: Negarestani
authors.forename: Abdorrasoul
authors.initials: A
authors.lastname: Malekpour
authors.forename: Mehran
authors.initials: M
authors.lastname: Hakimzadeh
authors.forename: Naghi
authors.initials: N
authors.lastname: Dara
keywords: Autoantibody; Autoimmune Hepatitis; Liver Function Tests; Liver Transplantation